The Heidenhain form of Creutzfeldt-Jakob disease (CJD) is a rare CJD variant with predominantly visual symptoms in the early stages. Clinical manifestations of metamorphopsia, hemianopia and Balint's syndrome correlate with the involvement of the posterior cortical regions. A 71-year old healthy and very active man was admitted because of impaired visual acuity, hemianopia, and gait disturbance progressing over one week. MRI found typical cortical hyperintensities in the occipital regions while rhythm slowing and sharp waves were seen in the occipital regions on EEG. Protein 14-3-3 was detected in the cerebrospinal fluid. Postmortem neuropathology revealed typical histopathological changes consistent with CJD. Moreover, we found deposits of phosphorylated tau protein in the limbic regions that met the criteria for primary age-related tauopathy (PART); representing an additional and interesting finding in our case.

• MeSH
• Creutzfeldtova-Jakobova nemoc diagnóza   patologie   MeSH
• diferenciální diagnóza MeSH
• difuzní magnetická rezonance MeSH
• elektroencefalografie MeSH
• fatální výsledek MeSH
• fosforylace MeSH
• komorbidita MeSH
• lidé MeSH
• mozek patologie   MeSH
• neurologické vyšetření MeSH
• poruchy zraku diagnóza   patologie   MeSH
• proteiny 14-3-3 MeSH
• proteiny tau chemická syntéza   MeSH
• senioři MeSH
• tauopatie diagnóza   patologie   MeSH
• týlní lalok patologie   MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH